Cardiac sarcoma’s are highly aggressive tumours. Clear resection margins ± autotransplant, followed by chemotherapy, offers the best survival chance. Therefore, frozen section should be preformed when there is ambiguity in diagnosis

Diab, MS

Fletcher, N

Sarsam, M

Smelt, J

PubMed

 Cardiac sarcoma’s are highly aggressive tumours. Clear resection margins ± autotransplant, followed by chemotherapy, offers the best survival chance. Therefore, frozen section should be preformed when there is ambiguity in diagnosis. </jats:p

Mohammad S Diab

Jeremy Smelt

Nick Fletcher

Mazin Sarsam

Crossref

JRSM Open

Cardiac sarcoma presenting with abdominal pain and mimicking myxoma on echocardiogram

St George's Online Research Archive

Case ReportCardiac sarcoma presenting with abdominal pain andmimicking myxoma on echocardiogramMohammad S Diab1, Jeremy Smelt1, Nick Fletcher2 and Mazin Sarsam11Department of Cardiothoracic Surgery, St. George’s Hospital, London SW17 0QT, UK2Department of Anaesthesia, St. George’s Hospital, London SW17 0QT, UKCorresponding author: Mohammad S Diab. Email: diab.mohammed@gmail.comLessonCardiac sarcoma’s are highly aggressive tumours. Clearresection margins autotransplant, followed by che-motherapy, offers the best survival chance. Therefore,frozen section should be preformed when there is ambi-guity in diagnosis.KeywordsCardiovascular medicine, surgery, oncologyIntroductionWe present an unusual case of an AMI due to super-ior mesenteric artery (SMA) embolus originatingfrom a left atrial sarcomatoid neoplasm mimickinga myxoma on echocardiogram. Acute mesentericischaemia (AMI) is associated with high mortalityand the usual culprits are emboli due to atrial ﬁbril-lation or following low cardiac output state.The patient underwent emergency embolectomyand bowel resection followed by cardiac surgery.Frozen section conﬁrmed an undiﬀerentiated sar-coma. Frozen section is necessary to identify malig-nant cardiac tumours and perform extensive tumourresection to obtain clear margin which dictatessurvival.Case presentationA previously ﬁt and well 45-year-old man attendedhis local hospital with abdominal pain. He had raisedinﬂammatory markers and serum lactate. AbdominalCT angiogram revealed a superior mesenteric arteryembolus and evidence of infarcted bowel.The patient underwent a laparotomy, superiormesenteric artery embolectomy and 30 cm of ischae-mic jejunum was resected and a primary anastomosiswas formed. The histology showed ischaemic bowelonly. Transthoracic echocardiogram showed thepresence of a large left atrial mobile mass attachedvia a pedicle, prolapsing through the mitral valvefrom the left atrium at 3.4 cm in length (Figure 1.)It was unclear whether this was a myxoma or athrombus. The patient’s ventricular function wasnormal and a coronary angiogram was reported asnormal. He was transferred to a cardiac centre andunderwent median sternotomy and aortobicaval can-nulation 17 days after his laparotomy. The heart wasarrested with anterograde and retrograde cardiople-gia. The mass was found to be densely adherent to theleft atrial free wall through to the right pulmonaryveins and the superior vena cava, which was thendetached from the right atrium for access.Ambiguity about the tumour’s nature made us senda frozen section specimen which revealed sarcoma-toid appearances. The mass was peeled oﬀ the anter-ior mitral valve leaﬂet and excised with the invadedstructures. The sinoatrial node had to be sacriﬁced.Bovine pericardium was used to reconstruct the leftand right atrium and right pulmonary veins(Figure 2). On coming oﬀ, bypass mitral regurgita-tion was observed via the transesophageal echo andso the neo left atrium was reopened and a 32mmCosgrove-Edwards ring implanted. The patient hadan uncomplicated and short hospital stay. Histologywas consistent with mildly pleomorphic spindle shapesarcoma supported by positive immunohistochemis-try for SMA and the presence of MDM2 gene amp-liﬁcation. Despite the extensive tumour resection andchambers reconstruction, there was tumour margininvolvement. The patient remains well ﬁve monthslater and is under the care of a sarcoma oncologyteam.DiscussionPrimary cardiac tumours are rare (<0.04%), butfatal.1 Malignant tumours constitute 25% of all pri-mary cardiac tumours, of which sarcomas comprom-ise >75%.1 Surgery achieves the optimal results fortreatment of localised sarcomas. Prognosis is depend-ent on radical tumour resection.2 There is still noclear deﬁned treatment guidelines for malignant! 2016 The Author(s)Creative Commons CC-BY-NC: This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 3.0 License (http://www.creativecommons.org/licenses/by-nc/3.0/) which permits non-commercial use, reproduction and distribution of the work without further permission providedthe original work is attributed as specified on the SAGE and Open Access page (https://uk.sagepub.com/en-us/nam/open-access-at-sage).Journal of the Royal Society of Medicine Open;0(0) 1–3DOI: 10.1177/2054270416649286 by guest on September 1, 2016shr.sagepub.comDownloaded from cardiac tumours. There is no gender preferencenoted, and presentation is usually in the fourthdecade of life.3 Three large series (reviewing 34, 21and 95 cases respectively) showed that overall mediansurvival was 12–20 months.3–5 Moreover, they alsoshowed that survival was 6–10 months in R1 resec-tion while that improved to 12–24 months in R0resection. Surgical mortality was reported to behigh (8.3%), albeit acceptable.4Due to the paucity of cases of this tumour, deﬁnitepathological or radiological diagnostic characteristicis challenging. Echocardiography detects cardiactumours with high sensitivity and speciﬁcity,2 butonly endocardial biopsy gives the deﬁnitive diagno-sis.2 Usually, the rapidly progressive clinical course,multiple tumour growths and non-septal attachmentof the tumour all suggest a malignant proﬁle of theprimary cardiac tumours. In our case, the tumourwas attached to the septum and at the same timeextending into the pulmonary veins. There was uncer-tainty in diagnosis and a frozen section conﬁrmed thediagnosis and immediate reconstructive surgery toclear the tumour was performed.As with any malignant tumour, we put the emphasison clear resection margin to provide the best disease-free survival. In two isolated case reports of left atrialsarcomas, the patients survived for 7 and 11 years.6,7In both reports, the patients underwent a re-operationfor local recurrence. Unsurprisingly, case series haveshown that R0 resection can be achieved in only38–42% of cases.3–5 Furthermore, emergency surgerywas performed for palliation or to prevent furtherembolism that can lead to partial tumour resection.Thus, local recurrence is certain. Therefore, chemother-apy should be almost always administered. There hasbeen controversy about oﬀering heart transplant, andinmany studies that showed no surgical advantage oversurgical resection chemotherapy.1,2 Also, there havebeen ethical issues about oﬀering heart transplant topatients with malignant cardiac tumours. There is nospeciﬁc guidance on chemotherapy regimens, but itseems thatmultiple chemotherapies are better and guid-ance should be aided by C-Kit sensitives.To conclude, endocardial frozen section is neces-sary when there is echocardiographic ambiguityabout the tumour nature. We recommend closefollow-up and chemotherapy in all cardiac sarcomas.Primary (intimal) spindle shape sarcoma is veryaggressive and R0 resection is diﬃcult to achieveand heart transplantation may be indicated whenFigure 1. Transesophageal echocardiogram demonstrating a wide base left atrial mass (arrow).2 Journal of the Royal Society of Medicine Open 0(0) by guest on September 1, 2016shr.sagepub.comDownloaded from metastatic workup is negative. A cardiac sarcomaregistry is required to obtain more solid managementguidelines.DeclarationsCompeting interests: None declaredFunding: None declaredEthical approval: Written informed consent to publication wasobtained from the patient’s next of kin.Guarantor: MSDContributorship: MSD did the literature search and wrote themanuscript. JS provided the images and revised the manuscript.NF and MS proof read and approved the ﬁnal manuscript.Acknowledgements: NoneProvenance: Not commissioned; peer-reviewed by Chung Lim.References1. Jimenez MJM, Fuentes Manso R, Segovia Cubero J,et al. [Is heart transplantation for primary cardiacsarcoma a useful therapeutic option?]. Rev Esp Cardiol2003; 56: 408–411.2. Uberfuhr P, Meiser B, Fuchs A, et al. Heart transplant-ation: an approach to treating primary cardiac sarcoma?J Heart Lung Transplant 2002; 21: 1135–1139.3. Ramlawi B, Leja MJ, Abu Saleh WK, et al. Surgicaltreatment of primary cardiac sarcomas: review ofa single-institution experience. Ann Thorac Surg 2016;101: 698–702.4. Putnam JB Jr., Sweeney MS, Colon R, et al. Primarycardiac sarcomas. Ann Thorac Surg 1991; 51: 906–910.5. Simpson L, Kumar SK, Okuno SH, et al. Malignantprimary cardiac tumors: review of a single institutionexperience. Cancer 2008; 112: 2440–2446.6. Clores MJ, Monzur F and Rajapakse R. Acute mesen-teric ischemia caused by rare cardiac tumor embolus.ACG Case Rep J 2014; 2: 27–29.7. Modi A, Lipnevicius A, Moorjani N and Haw M.Prolonged survival with left atrial spindle cell sarcoma.Interact Cardiovasc Thorac Surg 2009; 8: 703–704.Figure 2. Intraoperative view. (a) Note that the tumour extends into and through the wall of the superior vena cava and the leftatrial dome; (b) Complete tumour removal by en bloc resection, including the right atrial anterior and lateral wall, left atrial dome,and a large segment of the superior vena cava; (c) Repair of the left atrial dome; (d) Reconstruction of the right atrium andsuperior vena cava with bovine pericardium.Diab et al. 3 by guest on September 1, 2016shr.sagepub.comDownloaded from 

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Cardiac sarcoma presenting with abdominal pain and mimicking myxoma on echocardiogram

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